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Biochem HLS > Lec 3 > Flashcards

Lec 3 Flashcards

1
Q

What is the prophyrins?

A

Cyclic compound consist of 4 pyrrole ring connection by methine bridges

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2
Q

What is the difference between porphyrins compounds?

A

Side chane and distribution of it

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3
Q

What is heme ?

A

Protoporphyrin IX (one of porphyrine compounds) with FE

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4
Q

Where biosynthesis of heme occurs ?

A

In liver (15%) & in erythrocytes (85%)

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5
Q

What is the enzyme that shared in first step of heme biosynthesis?

A

Aminolevulinic acid synthase 1

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6
Q

What is the precursor of aminolavulinic acid ?

A

Glycine & succinyl coA

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7
Q

What is the products of first step of heme biosynthesis?

A

Aminolevulinic acid & coA & co2

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8
Q

What is the rate limiting step in heme biosynthesis?

A

Aminolevulinic acid synthase

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9
Q

Does heme affect ALAS1?

A

Yes inhibit it

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10
Q

What is the enzyme that lead inhibit it in heme biosynthesis?

A

Aminolevulinic acid reductase & ferrochelatase

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11
Q

What is the porphyria type I

A

Deficiency in utoporphyrinogen III synthase

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12
Q

What is the enzyme that convert protoporphyrin IX to heme ?

A

Ferrochelatase enzyme which add FE to protoporphyrin

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13
Q

Ferrochelatase enzyme found in ?

A

Mitochondria

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14
Q

Aminolevulinic acid synthase found in ?

A

Moitochondia

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15
Q

How I can detected the deficiency of FE?

A

By elevated the zinc protoporphyrin which zinc bind to protoporphyrin instead of FE

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16
Q

What is the porphyria?

A

Porphyria refers to a group of disorders that result from a buildup of natural chemicals that
produce porphyrin in the body.

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17
Q

What is the categories of porphyria?

A

Acute (affect CNS) cautaneous (affect the skin)

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18
Q

Which one of porphyria is recessive ?

A

Congenital eythropoietic porphyria

19
Q

What is the result of prophyria?

A

Formation of superoxide radical , skin blister, skin darken

20
Q

What is the most common porphyria ?

A

Porphyria cutanea tarda

21
Q

What is the cause of porphyria cutanea tarda ?

A

Deficiency in uroporphyrinogin decarboxylase enzyme

22
Q

What is the function of uroporphyrinogen decarboxylase?

A

Convert uroporphyrin to coproporphyrinogen

23
Q

What is the result of porphyria cutanea tarda?

A

Acummulation of uroporphyrogen in urine which lead to converted the color of urine to red , and lead to red to brown skin eruptions

24
Q

What is the significant of heme degradation ?

A

Prevent heme toxicity
Excretory system
Product of degradation act as messenger products
Response to oxidative stress

25
Q

What is the enzyme that act on first step of heme degradation?

A

Heme oxygenation

26
Q

The first step of heme degradation need NADPH or NADH or FADH2?

27
Q

What is the compound that result from first step of heme degradation?

A

Biliverdin & co & FE+3

28
Q

What is the enzyme that convert biliverdin to bilirubin?

A

Biliverdin reductase (need NADPH)

29
Q

Synthesis of bilirubin occurs where?

A

In macrophages

30
Q

What is the protein that bilirubin bind with it in blood

31
Q

What is the conjugation form of bilirubin?

A

Bilirubin diglucuronide

32
Q

Which enzyme convert bilirubin to bilirubin diglucuronide

A

Bilirubin glucuronyl transferase (needed 2 glucornic acid)

33
Q

What is the color of bilirubin?

A

Red-orange

34
Q

What is the effect of salicylate & sulfonamide on bilirubin?

A

It replaces it in bilirubin which lead to enter CNS and neural damaged

35
Q

Deficiency in bilirubin glucurnyl transferase lead to ?

A

Gilbert syndrome & crigler najjar

36
Q

Deficiency in enzyme that transport conjugated bilirubin out the liver lead to

A

Dubin-Johnson syndrome

37
Q

How urobilinogen synthase ?

A

Bacteria reduced bilirubin diglucuronide by remove glucornic acid

38
Q

What is the compound that secreted to feces (final compound of bilirubin) and what is the color of it and how it production?

A

This compound is sterocobilin and produce by oxidized urobilinogen and have brown color

39
Q

Urobilinogen or sterocobilin secreted by urine and which one by feces?

A

Urobilinogen by iron & sterocobilin by feces

40
Q

What is the juandice ?

A

Yellow color of skin , nail bad & sclera

41
Q

He have 3 type of juandice, mention them and what is the type of bilirubin increase during this ?

A

1-prehepatic juandice, which lead to increase in unconjugated bilirubin
2-hepatocellural juandice, which lead to increased in both conjugated & unconjugated bilirubin
3-posthepatic or cholestatic juandice, which lead to increase in conjugated bilirubin

42
Q

What is the kernicterus?

A

Damaged to brain due to accumulation of bilirubin in brain

43
Q

By any type of bilirubin kernicterus can occurs ?

A

By unconjugated because it is water insoluble so it can bass B.B.B

Biochem HLS (3 decks)

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